Ondine's curse: a discussion of five cases

Abstract

Five cases of central congenital hypoventilation, Ondine's curse, were studied. The diagnostic criteria were: lack of respiratory autonomy during sleep, abnormal CO2 test results and abnormal respiratory monitoring results during sleep. This hypoventilation induced chronic complications, such as a pulmonary arterial hypertension and psychomotor and/or growth retardation. The other conditions frequently associated with Ondine's curse were: brainstem disturbances, Hirschsprung disease and neuroblastoma. Since other brainstem dysfunctions are sometimes associated with hypoventilation and because complications can arise, these children routinely underwent complementary investigations. Treatment was symptomatic. Assisted mechanical ventilation was initiated as soon as possible and carefully monitored. The prognosis for these children has greatly improved and some of them lead a normal life with nightly assisted ventilation at home.